Surgical treatment of pulmonary artery primary myxosarcoma

Case of the 47-year-old man, clinically suffering from recurrent dyspnoea, cough, syncopes, haemoptysis, unsuccessfully treated with anticoagulant and fibrinolytic agents, is described. Large masses of a tumour, occupying nearly the whole lumen of pulmonary artery and extending to peripheral branches, were removed during surgery. The tumour was histologically and immunohistochemically classified as myxosarcoma. Following surgery, the patient was asymptomatic, could do his original job (lorry-driver) for the next 14 months. He died 20 months after surgery, showing symptoms and signs of progressive right heart failure. At autopsy, the tumourous obliteration of the pulmonary artery and adjacent thrombosis were found. Primary pulmonary artery sarcoma should be considered in the differential diagnosis of recurrent pulmonary thromboembolism, especially when no peripheral vein thrombosis could be found.